therefore, are generally safe even in individuals with

ulcerative colitis who are on immunosuppressive therapy. All patients with ulcerative colitis should receive
an annual vaccine for inuenza. All patients with ulcerative colitis should be considered for an annual
vaccine for inuenza.
Live viral vaccines should be avoided in immune compromised children. This includes children on steroids
(prednisone >20mg/d or 2mg/kg/day for 2 weeks
or more), 6 MP/azathioprine, or methotrexate.
Whenever possible, serologic conversion should be
documented in children being immunized while immune compromised.

When should I be concerned that the ulcerative

colitis is aring?
It is an old adage that patients are true. The symptoms the child had at rst presentation are generally
the same symptoms at time of are. Rectal bleeding
would be the most common symptom of a UC are.
Other potential symptoms include diarrhea, fever,
persistent abdominal pain or extra-intestinal symptoms including a rash, arthralgias/arthritis, jaundice
or eye pain.

rectum or pouch. The ultimate result allows for continuity

of the gastrointestinal tract without a permanent stoma. An
inammation of the pouch can develop (pouchitis) and this
can usually be treated medically.

How important is cancer surveillance

in a UC patient?
It has been well established that patients with UC have an
increased risk of colo-rectal cancer. Regular colonoscopy
for cancer surveillance is recommended for all patients who
have had UC more than 8-10 years including pediatric and
adolescent patients. Surveillance of the pouch in patients
who have undergone colectomy is usually recommended
as well.

CHALLENGES IN
PEDIATRIC ULCERATIVE COLITIS
ADVICE TO THE PRACTITIONER

Diagnosis
and Management of
Ulcerative Colitis

Physician Notes

When is it not a disease are?

It is not always easy to distinguish between a viral
illness and a are of the ulcerative colitis. Time,
patience and resolution of symptoms are often the
determining factors. It is important to consider other
sources for an illness prior to ascribing symptoms
to the ulcerative colitis. Stool cultures and screening
blood work looking for anemia, hypoalbuminemia
or elevation of acute phase reactants can be helpful
as well. Patients and their families can often tell the
difference between an intercurrent illness and a are
of the ulcerative colitis.
First line anti-pyretic and analgesic therapy (for common fever, headaches and the like) should probably
be acetaminophen since there is some data to suggest that chronic NSAID therapy may adversely affect UC.

What is the role of surgery?

While medical therapy is the mainstay of treatment, surgery is performed in as many as 25% of pediatric UC
patients. Steroid dependence and lack of response to
medical therapy are the most common indications for
colectomy in UC.
Advances in surgical techniques have made it possible
to remove the colon and use the terminal ileum as a neo-

www.KidsIBD.org
www.CDHNF.org
www.NASPGHAN.org
CDHNF National Ofce:
P.O. Box 6, Flourtown
PA 19031
Phone: 215-233-0808
Educational support for The CDHNF
Pediatric Ulcerative Colitis Campaign
was provided by Procter&Gamble

CHILDRENS DIGESTIVE
HEALTH & NUTRITION
FOUNDATION

NORTH AMERICAN SOCIETY FOR

PEDIATRIC GASTROENTEROLOGY,
HEPATOLOGY AND NUTRITION

CHILDRENS DIGESTIVE
HEALTH & NUTRITION
FOUNDATION

NORTH AMERICAN SOCIETY FOR

PEDIATRIC GASTROENTEROLOGY,
HEPATOLOGY AND NUTRITION

an attractive choice especially in patients who are ill and

have a poor quality of life at the time of diagnosis. The
immunosuppressive, cosmetic and metabolic side effects
of steroids make them inappropriate to use as maintenance agents.

What is pediatric ulcerative colitis?

Ulcerative Colitis (UC) is an auto-inammatory process that
results in chronic inammation of the large intestine. UC
involves only the mucosa and extends proximally from the
rectum. In children and adolescents, the area of involvement often extends beyond the splenic exure. The extent
of involvement does not correlate well with clinical disease
activity. The mucosal depth and continuous distribution of
the inammation distinguishes UC from Crohns disease in
most cases.

5-aminosalicylic acid (5-ASA) preparations are effective anti-inammatory agents and have been demonstrated to both induce and maintain remission in UC.
Clinical response can be somewhat slower than steroids
and, therefore, patients with severe UC continue to receive steroids in most cases. Once remission is achieved,
either with 5-ASA or steroids, maintenance therapy with
5-ASA is the most common therapeutic approach. There
are oral and rectal preparations available.

Most researchers believe that UC is caused by a combination of genetic and environmental factors. Although UC
may occur at any pediatric age, it is most often diagnosed
in early adolescence. It affects males and females equally.

How does ulcerative colitis present?

The most common presentation of a patient with UC is
bloody diarrhea and abdominal cramping. Extra-intestinal
symptoms may also be present such as fever, skin rash, joint
symptoms including frank arthritis, and liver disease.

What evaluation should be offered by

the primary health care provider?
Cultures for bacterial pathogens (Salmonella, Shigella,
Campylobacter, Yersinia, E. Coli including 0157:H7,
Clostridium difcile toxins A & B) should be completed.
Lab testing for parasites including serology for Entameba
histolytica should be considered.
Additional testing (Complete blood counts with differential,
sedimentation rate, C-reactive protein and serum chemistries including albumin) should be part of the primary evaluation of the patient with persistent rectal bleeding. Anemia,
elevation of acute phase reactants and hypoalbuminemia
may be present indicating inammation and chronicity. If
the cultures are negative and the symptoms persist beyond
two weeks, then referral to a pediatric gastroenterologist is
appropriate.

When should I refer to a pediatric

gastroenterologist?
Patients presenting with more insidious intermittent rectal
bleeding, additional factors such as weight loss; extra-intestinal manifestations of inammation and a family history of
IBD should prompt early evaluation and referral.
In patients where the infectious evaluation is negative but
bleeding persists with or without pain, referral is indicated.

How is the diagnosis made?

The diagnostic gold standard is a colonoscopy with biopsy. Grossly, a continuous colitis that begins in the rectum
is the most common appearance of UC. Biopsies are taken
to conrm the diagnosis and further exclude infections.
It is not unusual for a pediatric gastroenterologist to also
perform esophagogastroduodenoscopy (EGD) at the
time of the colonoscopy since the EGD can help classify the
diagnosis as one of Crohns disease or UC. Additonally,
a contrast small bowel series is frequently performed
to image the rest of the bowel and to conrm that all the
involvement is restricted to the colon and does not involve
the small intestine, as is seen in Crohns disease.
More recently, it has been noted that there are various antibodies that are seen in a majority of individuals with inammatory bowel disease (IBD). While the diagnosis of IBD
cannot be made serologically, antibody testing can help
to conrm the diagnosis. Peri-nuclear anti-neutrophil cytoplasmic antibody (pANCA) is an auto-antibody that is most
associated with UC and may help predict some potential
long-term complications.

What treatments should I expect my

patient to receive?
As with any chronic condition, the rst goal of medical therapy in UC is to induce a clinical remission. Once remission
is achieved, therapy is directed to maintain that remission.
Corticosteroids have been the mainstay of induction therapy for many years. The rapid onset of action makes steroids

Recurrent use of steroids becomes a concern in patients

who are intolerant or unresponsive to 5-ASA. Since a
goal of therapy is to eliminate long-term steroid use, immunomodulator therapy may be introduced. 6-mercaptopurine (6MP) and its parent compound Azathioprine are
the immunomodulators most commonly used to steroidspare in pediatric UC.
Most recently, biologic therapy in the form of iniximab
(Remicade) has been approved for the treatment of refractory UC in adults.
In addition to medical therapy, psychosocial support of
the pediatric patient and the affected family is critical in
UC or any chronic pediatric illness. Implementation of a
psychosocial support plan is a critical component of the
health care plan. Often, the primary care provider has
a unique and long term relationship with the family and
child. This relationship can facilitate the execution of a
coordinated psychosocial support plan

What are primary care issues

in ulcerative colitis
How important are vaccines?
It is important to recognize that children with ulcerative colitis continue to be at risk for routine childhood
illnesses. Immunizations are an important protective
mechanism and children and adults with ulcerative
colitis should be maintained on the recommended immunization schedule.
At diagnosis, it is important that the immunization history be reviewed so that catch-up immunizations can
be given if needed. Immunity to varicella should be
conrmed by history or serology so that those who
require varicella vaccine can receive the appropriate
doses prior to receiving immunosuppressive therapy.
The majority of vaccines do not contain live virus and,